Pseudotumor cerebri syndrome (also described as cerebri) is a disorder that promotes an increase in intracranial pressure and papilla edema in the absence of hydrocephalus or structural brain lesions and cerebrospinal fluid without changes in its composition.
It can usually occur in its primary form whose etiology is undefined, also known as idiopathic intracranial hypertension. However, it can be due to other secondary causes, such as: medications, vascular changes, sleep apnea and anemia.
The magazine cephalalgia published a prospective study whose objective was to compare patients with pseudotumor cerebri syndrome of idiopathic and secondary causes in order to detect whether there are clinical or neuroimaging factors that may point to these secondary causes. The comparison of these groups was performed with the phenotypic evaluation of the patients, clinical manifestations and neuroimaging.
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The cohort study was observational and prospective. The inclusion criterion was patients with clinical suspicion of onset of pseudotumor cerebri syndrome over 18 years of age. The exclusion criteria were as follows: pregnant or lactating women, patients who did not meet Friedman’s revised criteria and lack of data in medical records.
The analyzed public was 278 participants with clinical suspicion of onset of pseudotumor cerebri syndrome were included, coming from two specialty centers (Odense University Hospital and Danish Headache Center) between January 2018 and July 2021.
All participants had diagnostic workup, such as: complete anamnesis, neurological and neuro-ophthalmological examination, routine laboratory review, rachicentesis with opening pressure measurement and routine cerebrospinal fluid, neuroimaging with magnetic resonance imaging of the brain and evaluation with venous vessel study.
After this workup, patients were diagnosed with pseudotumor cerebri syndrome and were divided into two groups:
- (1) patients with idiopathic intracranial hypertension (IIH)
- (two) patients with secondary pseudotumor cerebri syndrome (sPTCS).
Patients with secondary pseudotumor cerebri were categorized into five causes: drug-related, systemic diseases, sleep apnea, cerebrovascular diseases, and miscellaneous (other causes that are known to predispose to sPTCS).
Neuroimaging evaluation was performed by a neuroradiologist. We were instructed to identify specific signs of increased intracranial pressure as described in Friedman’s revised criteria: sella turcica emptying, flattening of the posterior aspect of the eyeball, distention of the perioptic subarachnoid space, and transverse venous sinus stenosis.
Among the 278 participants initially included, 148 were actually analyzed for this study in which 120 were patients with idiopathic intracranial hypertension and 28 were patients with secondary pseudotumor cerebri. The remaining 130 participants were excluded because they did not meet Friedman’s revised diagnostic criteria (n = 95), disease recurrence (n = 12), pregnancy (n = 3), and lack of data (n = 20).
The most prevalent causes were medication (n = 8; 28.6%) and systemic causes (n = 8; 28.6%). The group of drug causes linked to PTCS included tetracycline, lithium, prednisolone, retinoids, tacrolimus, and ustekinumab. The group of systemic causes included cases such as systemic lupus erythematosus and anemia.
As for the other categorized groups, 17.9% were related to sleep apnea, although only 32 participants included in the study underwent polysomnography. In addition, 14.3% have cerebrovascular causes such as cerebral venous thrombosis (CVT), stenosis after previous CVT, and antiphospholipid syndrome (even without confirmation or suspicion of thrombosis).
In both groups, most participants were female (92.9% in SPTCS and 96.7% in IIH, p=0.3) and obese (BMI 35.6 versus 36.4 respectively, p=0.6). In both groups, the mean age group was in the fertile period, and it is worth mentioning that patients with sPTCS were older when compared to IIH (mean age of 33.9 years). versus 28.9 years, respectively, p=0.003).
The reported clinical manifestations were similar between the groups, which included chronic headache (with more than 15 days of pain per month), vertigo, transient visual obscuration, blurred vision and diplopia. Patients with IIH tended to have more tinnitus pulsatile (56.1%) when compared to patients with SPTCS (30.8%) with p-value = 0.03.
No significant difference was found in the severity of optic papilledema between the groups, although patients with sPTCS were more likely to have the syndrome without papilledema (18.5%) compared to patients with IIH (5.1%) with p-value = 0.04.
All participants in this study had a brain MRI and most had a study with venous vessels (96.4% of patients with sPTCS and 100% of those with IIH). There were no significant differences in any neuroimaging findings between both groups.
Identifying features that differentiate secondary causes of pseudotumor cerebri syndrome for IIH are important as they change the therapeutic approach. In this prospective observational study, it was observed that the most described secondary causes, in order, were: medications, systemic diseases, sleep apnea and cerebrovascular diseases.
Although some significant differences were identified between the groups, such as age and frequency of tinnitus pulsatile, these characteristics do not help the clinician in his practice to discern between idiopathic or secondary causes of pseudotumor cerebri. It has been shown that patients with SPTCS were more likely to be diagnosed with no evidence of optic papillary edema when compared to those with IIH – this finding may suggest that patients with pseudotumor cerebri syndrome without papilledema may have secondary causes and should undergo a more thorough workup. extensive to remove them.
An interesting finding is that clinicians, in general, should be very cautious when excluding secondary causes of pseudotumor cerebri in any patient who meets diagnostic criteria for such. This information is due to the fact that 82.1% of the sample of this study with secondary causes of pseudotumor cerebri required additional treatment beyond what is already standardized for this condition.
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Secondary causes of pseudotumor cerebri should be investigated in all patients suspected of having this syndrome. This prospective observational study demonstrated that both idiopathic and secondary causes present patients who are phenotypically and clinically similar.
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